Der Gerinnungsfaktor XIII – Pathophysiologie, Klinik und Therapie von Mangelzuständen

Zusammenfassung

Die Transglutaminase Faktor XIII (FXIII) besitzt ein vielfältiges Wirkspektrum, das zentrale Funktionen in der sekundären Hämostase einschließt. Multifaktoriell bedingte FXIII-Mangelzustände können u. a.mit Aborten, Wundheilungsstörungen und lebensbedrohlichen Koagulopathien assoziiert sein. Die vorliegende Übersichtsarbeit beschreibt die physiologischen Funktionen von FXIII sowie Pathophysiologie, Diagnostik und Therapieoptionen von FXIII-Mangelzuständen.

Abstract

The complex activity of the transglutaminase factor XIII (FXIII) comprises central functions in secondary hemostasis. Congenital or acquired FXIII deficiencies may be associated with habitual abortions, impaired wound healing, coagulopathy and fatal hemorrhage. The present review describes physiological functions of FXIII, as well as pathophysiology, diagnostic and therapeutic options of FXIII deficiencies.

Kernaussagen

• Faktor XIII (FXIII) nimmt als Transglutaminase eine Sonderstellung unter den Gerinnungsfaktoren ein.

• FXIII besitzt geringe Substratspezifität. Sein Wirkspektrum schließt Funktionen bei der Hämostase, der Wundheilung, der Aufrechterhaltung einer Schwangerschaft und der Immunmodulation ein.

• Kongenitale heterozygote Mangelzustände haben eine relativ hohe Prävalenz (ca. 1 : 1000) und sind klinisch häufig inapparent.

• Sekundär erworbene Mangelzustände sind oft unspezifische Komorbidität internistischer Erkrankungen oder Folge von Blutverlust, Verbrauch und Dilution.

• FXIII-Mangel kann mit Koagulopathie und lebensbedrohlichen Blutungen assoziiert sein.

• Konventionelle Laborgerinnungsdiagnostik oder Point-of-Care-Verfahren wie die Thrombelastometrie erlauben keine Aussage über die FXIII-Aktivität.

• Therapieoptionen bestehen in der Transfusion von Frischplasma bzw. der Applikation von pasteurisierten oder rekombinant hergestellten Faktorenkonzentraten.

Schlüsselwörter:

Faktor XIII - Gerinnung - Hämostase - Faktor-XIII-Defizit - Koagulopathie

Keywords:

factor XIII - coagulation - hemostasis - factor XIII deficiency - coagulopathy

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